Part 1: Seriously? Where do I begin? Stevens-Johnson Syndrome…

I shake my Fists at You!

Kyle in the hospital

Over the next few weeks I want to share our story, the story of how Stevens-Johnsons Syndrome came into our life (twice), burned our house down and changed us forever. Our world completely turned on its head, I honestly do not know how we made it through. Truth be told, we are still not out of the woods and sweet Kyle will be dealing with the physical complications (like a very weakened immune system) for the rest of his life. Just this week he is getting over the combo-illness-package of Hand Foot & Mouth, Strep Throat & a nasty Staph Infection on his upper thigh. I am so glad he did not have to go back in the hospital. That’s how he rolls — now.

Dave, Eli and I are still dealing with the residual effects of being passengers along for this crazy, mean and long ride. I still wonder if Eli, who was so neglected, really knows that we love him just as much as we love his big brother.

Eli & Me

What I can tell you is that we are stronger and definitely more focused. The little things just don’t seem as big anymore. Kyle is one tough kid with a pain threshold that is beyond anything I have ever seen. In October 2010 Kyle was admitted to Primary Children’s Medical Center a very sick young man, with his face swollen, eyes red like I have never seen and ironically with Pneumonia. As it states on its Website, Primary Children’s Medical Center serves the needs of children in the states of Utah, Idaho, Wyoming, Nevada and Montana. The hospital is equipped to treat children with complex illness and injury and is recognized as one of the top children’s hospitals in the United States. We knew this was the best place Kyle could be. We knew we were in good hands except for one little glitch. See, from October – November Kyle’s doctors spent a lot of time trying to figure out what was wrong with Kyle, often likening Kyle’s experience to an episode of House:

“>Interestingly, in last week’s House episode, after a diagnosis of Stevens-Johnson syndrome, House called it a day and let the team go home. With no known cure, it was odd to see House just give up. I’m not sure if it was him accepting defeat or what, but it seemed oddly out of character.

Stevens-Johnsons Syndrome Lips

The doctors could never quite agree (that is a whole other part of the story) on what Kyle actually had, which consequently affected Kyle’s treatment and long term healing process. (Yes, let me be absolutely clear. The diagnostic disagreements between the doctors made things a lot worse.) Because they had at least agreed on a family of diseases, I, of course, was on the internet trying to find anything I could to prove to them exactly what the first doctor who saw Kyle diagnosed him with. Because this initial attending physician is a dermatologist and this family of disease is referred to as “the only reason a dermatologist is called to the ER,” I felt confidant in the initial diagnosis. Kyle’s case was more rare, which made me slightly more patient with his doctors. See, Stevens-Johnsons Syndrome affects the mucous membranes of the body. Usually the epidermis is affected often sending a patient to the burn unit. Kyle’s SJS went internally. It started with his eyes, nose and mouth and moved down his throat and through his digestive system. We still do not know how permanent his lung damage is. Because there was continued disagreement, one of the many things I did to advocate for Kyle was research. I researched things like TENS, Erythema Multiformae Major and Minor. Through a month long stay in the hospital and finally with a simple biopsy, it was concluded that Kyle, in fact, had Stevens-Johnsons Syndrome. To this day, I think I would have rather he had been bitten again by a Copperhead Snake.

Kyle

[to be continued]

Here are a few things I jotted down during our month long hospital stay:

The role of a parent: Diplomacy, Advocating & Educating. Mini Update: Just watched the Ophthalmologist numb Kyle’s eyes and clean them with several surgical swabs and then this metal tool to break up eye adhesions. – 11.04.10

No Drama Just Truth: Now that we are coming up for air I can begin to express the state of absolute shock our family has been in for the past two weeks. The pain has been so incredibly difficult for Kyle that he was unable to articulate it in “traditional” ways. He is also good at putting on & then crashing when you leave the room. – 11.07.11

FROM my sister Brenda: Take a breath. You can do this. I so wish I could be there with you. We love you and will keep sending hugs and prayers. You guys are awesome parents. Kyle would not be getting the care he is with out you advocating for him. 11.07.11

Middle of the Night: What a roller coaster. It is nearly 1 AM. Kyle woke up in such pain. He is super congested & uncomfortable. After some tears, screams & pain medicine, he is calming down — I hope. Remembering what his Primary Dr. Says: healing is SLOW! – 11.06.11

. . . If you do not read any further and if you only take one thing away from this post, please take this. Tune out the noise and listen to your heart. Remember to advocate for your children and loved ones. Remember to get the doctors on your side and remember to be thoroughly vigilant. Trust yourself! No one will have their back like you!

Wow, Beth, just WOW. Kyle is truly an amazing kid to endure that, and as his parents (and little brother!) are equally as strong to have advocated and supported him throughout this. Although SJS is rare as you say, it’s frustrating to think Kyle had to suffer until a diagnosis was confirmed. Keep the faith!
P.S. I think you are doing exactly the right thing by sharing about this and educating us. I have a friend who also has a son with a very rare disease and she has chosen the same route…to spread the word. Thank you.

Seriously? You are always so thoughtful with your responses. Thank you! I think you are singlehandedly keeping me going. I applaud your friend and her son. What I learned is that no matter what the issue: sickness, school, emotions . . . , advocating with grace is key. As parents, it is our job. Thank you for getting it.

November 9, 2011

brenda

I believe I saw the SJS episode of House. I was shaking my fists at the TV. To me they down played the seriousness of SJS.
I am grateful you are educating others about SJS.
And, again , my hat’s off to you

July 9, 2012

Sammy

As a person who has suffered SJS myself a number of times I feel your pain. When I was 20 years old I had sinusitis, and took an over the counter cold and sinus medication. The next day when I woke up my eyes, nose, and mouth felt like they were on fire. When I first went to the doctor I was diagnosed with the coxsackievirus, and was given some “magic mouthwash” some lidocaine, and some darvocet to help with the pain. Unbeknownst to me I was actually having a sever allergic reaction to the pseudoephedrine in the cold and sinus medication I had previously taken. A few months later summer came around and my allergies started acting up, and my doctor told me to take a decongestant and so I did, still not knowing that I was allergic to pseudophed. I again went through the symptoms of SJS, but this time they were much worse, and I was bed ridden for a week, and only got relief when I took the pain meds I was prescribed, and was only able to drink when I first rinsed my mouth with the lidocaine, and even then everything tasted like blood. The doctors I went to were baffled. Eventually I was told to go see a dermatologist, and the dermatologist did a biopsy, and told me I had erythema multiforme, and that it was most often caused by the herpes virus. So for about a year I thought I had herpes, and was prescribed antivirals to help with the “outbreaks.” Well even on the antivirals I was continuing to have the problems, and never thought it was because of medicines I was taking because I was told I had herpes. I wanted a second opinion, and went to a different dermatologist. This one sat down with me and talked about my history, and finally figured out that although it was indeed erythema multiforme, that it was actually caused by a rare condition known as Stevens Johnson Syndrome. After knowing what caused it I stayed away from the meds that caused it, and didn’t have a problem for almost 2 years, although the damage that had been done because of the multiple times I had had SJS had caused me to have sometimes sever joint pain, and headaches. Unluckily for me I was hypersensitive to certain medications, especially medications that had been known to cause SJS. Because of my headaches I went to a neurologist, who prescribed my imitrex, and paxil. The paxil was for prophylactic measures, as it had been known to cause migraines and other headaches to occur with less frequency. After taking just one dose of paxil and one imitrex about 10 minutes later I started having the symptoms of SJS all over again. This time I was hospitalized for 2 weeks in the burn unit. Like Kyle however, my condition had mainly been inward, and I had fewer exterior symptoms before this time. The way the doctors explained it to me was that basically you burn from the inside out. This time was the most horrific pain I had ever felt, and this time also was the straw that broke the camels back. I was diagnosed with degenerative arthritis in my back hips and knees, because of the all the damage my body had been through. This was why I had had severe joint pain even after the other symptoms had gone away. I am sorry for the length of this comment, but it has been a while since I have been able to talk with someone who knew what I had been through. I wish Kyle the best of luck in the future. It has been a few years since the last time I had it, and my immune system is still not functioning the way it used to. Tell him to take plenty of vitamin c if he isn’t already. Anyway if anyone ever reads this and wants to respond feel free.

November 20, 2012

Kim

Hi. Your story is remarkable and so is your strength. You are in our thoughts and prayers. I’m grateful you had shared your sons story. I’m hoping you can help me… My daughter’s best friend is in the hospital and has just been diagnosed with Steven Johnson Syndrome. Our families are very close. The family is in shock over how sick this disease has made their daughter. She seems to have gotten worse since she has entered the hospital. The internet is overwhelming with information. You seem so knowledgable and helpful, having been through this. Could you please recommend a valuable resource my friend can turn to for information? I want to help her learn what is to be expected from this disease, what to ask the doctors, and what’s the best treatment for her daughter. I believe that knowledge is power, right now my friend is powerless, scared, and shocked. I’m trying to help her do some research. Thank you very much!!!!

Thank you for your feedback! I need to write even more about Stevens-Johnsons Syndrome. I agree. There is so little out there. I am so sorry to hear about your daughter’s best friend. How is she? I can imagine her shock and I am so sorry you all have to experience this. As far as a valuable source, that is a tough one. Wikipedia (which I am sure you have seen) probably has the best information. Any dermatology websites may have additional information. I know I needed information too and was very let down. Please encourage your friend to ask questions and to trust her instincts. She knows her daughter better than anyone. Please feel free to search SJS here and see what I have written. I have linked to other informative web sites as well. Good luck! Please know I am here if you have any more questions. All the best.

~Beth

September 8, 2013

Sarah Running

Beth- As, a 35yrold woman, and RN had known little about this disease. Until, I took the trip of a lifetime, a yoga trip to Bali. Getting sick on the other side of the globe was scary. Was very blessed to get home and diagnosed, and taken off the Lamictal relatively quickly. However, little is still understood with this horrible syndrome. SJS. I thought I was healing, and now it is attacking other parts of my body. I hope Kyle is now feeling better. I thought I knew so much about chronic pain, having chronic migraines that have only worsened since age six. But, it is nothing compared to the burning, searing, pain of SJS.
So, far I am “lucky”, was able to remain out of hospital. It looks as though this syndrome affects people differently. Now have the painful swollen mouth, tongue, with soles of hands and feet on “fire”. Which as a NICU RN has greatly effected my job 🙁 Hoping for light at the end of this fiery ugly tunnel. Sarah

February 2, 2014

jeanie digal

oh, what a meracle coming into his life. anyway i have some important question now because the son of my employer here in taiwan is having steven-johnson syndrome also. his ears become redness and also his lips. and now his suffering pains because of the wounds in his legs. can you help me on how to do medical treatment about this condition.

i will wait the response . thanks.

miss jean

November 27, 2016

Evie

Hello, how is your son? My son also has SJS. He is 16 & has suffered his entire life. I believe via a vaccine. We’ll never know. It’s heartbreaking. He worships good health & is so determined but sometimes the pain wins. Currently, it feels as though he has never been well. Seeing the terrible photograph of your sons lips is so upsetting. Since Drs wrongly diagnosed my son & prescribed Colchicine for 5yrs (nsaid) he dupidnt need, my son became addicted & allergic & the result is as with your son and also hands. Flares happen for no reason & currently the recent one is on week 9. He sees no “expert” as they really don’t know what they are dealing with. It took 16yrs to diagnose & I feel your pain. I like you just want one wish to be granted. Sorry if I’m not making sense. My brain is a jar of pickled research & fact gathering. Presently my son is taking zinc & Vit c. We are in London.

Hi there. Your comment disappeared for nearly a year. I am so sorry to hear about your son and his SJS. I am hoping things are better. Know you are not alone. xo

October 4, 2017

Heather

I may have overlooked this, but did the doctors say what caused your son’s SJS Syndrome to come on? My daughter has had “mild” symptoms for the past 2 days, and the pediatrician said hers was brought on by a combination of several meds she is taking. As she lies in the bed tonight with a severe headache, swollen red face and eyes, and the feeling of her throat closing in like she can’t breathe I pray that we don’t end up in the ER.

Hi Heather. The doctors think it was Advil. At first they thought it was mycoplasma Pneumonia. The truth is that the Pediatrician doe NOT KNOW what brought on her symptoms. They can only make an educated guess. Symptoms typically progress. I would take her back in. I would also ask to see a dermatologist. SJS and its variants are their expertise. She sounds a lot how Kyle was. Good luck. Let me know how it goes. Don’t be afraid to advocate for her. If it gets any worse, you may suggest they biopsy it.